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Startle seizures belong to reflex epilepsy syndromes. They usually occur in patients with mental deficiency and showing widely extended cortical lesions, often involving the sensorimotor area. Here we report three cases who did not fulfill these criteria, and in whom stereotactic electroencephalography (SEEG) recordings demonstrated the prominent involvement of the supplementary motor area (SMA). Visual analysis was complemented by time-frequency analysis of SEEG signals using a neuroimaging approach (Epileptogenicity Maps), which showed at seizure onset a significant increase of high frequency oscillations (HFOs, 60-100 Hz) over the premotor and prefrontal areas. Critically, in all cases, the SMA showed ictal HFOs at seizure onset and was included in the surgical resection. All patients became seizure-free after surgery, and histopathological examinations showed no specific lesion. These cases suggest the prominent but not exclusive role of SMA in startle seizures, and highlight the fact that surgery can be considered even in the absence of any magnetic resonance imaging (MRI) lesion., (Wiley Periodicals, Inc. © 2014 International League Against Epilepsy.)

We studied the effects of vagus nerve stimulation (VNS) on eating seizures, which theoretically would be triggered by neural activity and signaling from organs innervated by the vagus nerve. Three adult patients with daily nonreflex and reflex eating seizures were studied; one patient also had hot-water seizures. One patient had bilateral polymicrogyria and two had normal magnetic resonance imaging (MRI) findings. All patients were submitted to VNS implantation and had at least 2 years of postimplantation follow-up. Final stimulation parameters were 2.0-2.5 mA, 500 micros, and 30 Hz. Eating seizures decreased 70-95% and nonreflex seizures decreased 0-40% after VNS. There was no improvement in hot-water seizures. VNS seems to be an especially useful treatment modality in patients with reflex eating seizures not amenable to resective surgery.

Purpose: To determine the ictal-onset zone of musicogenic seizures by using intracranial EEG monitoring., Methods: Musicogenic seizures in three patients with medically intractable musicogenic epilepsy were first localized by using noninvasive methods including, in one patient, ictal magnetoencephalography (MEG) and magnetic resonance spectroscopy (MRS). The ictal-onset zones in these patients were then further localized using by intracranial EEG monitoring, and the outcomes of the two patients who underwent epilepsy surgery were determined., Results: Patient 1's musicogenic seizures localized to the right lateral temporal lobe, patient 2's originated in the right mesial temporal lobe, and patient 3's arose independently from both mesial temporal lobes. Patients 1 and 2 underwent resective epilepsy surgery and are seizure free (Engel class I)., Conclusions: Musicogenic epilepsy is a heterogeneous syndrome with seizures that can arise from multiple temporal lobe foci. Patients with medically intractable musicogenic epilepsy and with unilateral ictal onset zones may be considered candidates for resective epilepsy surgery.

Purpose: Malformations of cortical development (MCDs) are usually highly epileptogenic, and their hyperexcitability could facilitate the occurrence of reflex seizures. We sought to characterize reflex seizures in patients with MCDs and refractory epilepsy., Methods: Clinical, electrographic, and neuroimaging data were reviewed in eight patients with MCDs who had reflex seizures reproduced during presurgical evaluation., Results: All eight patients had both reflex and spontaneous seizures. In six, however, drop attacks or axial myoclonic seizures occurred only upon specific sensory stimulation. Reflex seizures were induced by more than one type of stimulus in most patients, but anatomofunctional correlations could usually be invoked. Six patients had significant intellectual impairment. Surgical resection controlled seizures in two patients., Conclusions: Reflex seizures in patients with MCDs may be medically refractory and may often manifest as drop attacks or axial myoclonus. Surgical resection of focal lesions can bring reflex seizures under control. Putative mechanisms related to the relatively low frequency of reflex seizures in MCDs are discussed.

Purpose: The neuropsychological approach to epilepsy is indispensable for assessment of cognitive function in an interictal period including pre- and postsurgical evaluation, and for disclosing the semiology of nonconvulsive status epilepticus. Another use of the neuropsychological approach is to identify a seizure-precipitating factor by loading systematic cognitive tasking, termed "neuropsychological EEG activation" (NPA), during standard EEG recordings., Methods: In this study, NPA tasks consisted of reading, speaking, writing, written arithmetic calculation, mental arithmetic calculation, and spatial construction., Results: The NPA tasks provoked epileptic discharges in 7.9% of the 480 epileptic patients and were often accompanied by myoclonic seizures. Among the cognitive tasks, mental activities mainly associated with use of the hands [i.e., writing (68.4%), written calculation (55.3%), and spatial construction (63.2%)] provoked the most discharges. Seizure-precipitating mental activities were found to be almost exclusively related to idiopathic generalized epilepsies (IGEs)., Conclusions: These results suggest that NPA is a useful tool for examining the relationship between cognitive function and epileptic seizures, and that the IGE patients with myoclonic seizures are vulnerable to higher mental activity.